Armaghan Ghareh Aghaji Zare; Afsaneh Radmehr; Sara Saniee; Mohadese Dalvand; Mohammad-Salar Hosseini
Abstract
Background and Aim: Autoimmune Bullous Diseases (AIBDs) are characterized by blistering skin and mucous membrane lesions. This study evaluated the quality of life and associated factors in patients with AIBDs.Methods: In this cross-sectional study, we included all clinicallyand laboratory-confirmed AIBD ...
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Background and Aim: Autoimmune Bullous Diseases (AIBDs) are characterized by blistering skin and mucous membrane lesions. This study evaluated the quality of life and associated factors in patients with AIBDs.Methods: In this cross-sectional study, we included all clinicallyand laboratory-confirmed AIBD patients older than 16 years who sought care at the Dermatology and Hair Clinic of Sina Hospital (Tabriz, Iran) from March to September 2020. We collected the demographic characteristics, disease profile, Autoimmune Bullous Disease Quality of Life (ABQOL) score, and Autoimmune Bullous Skin Disorder Intensity Score (ABSIS). The recorded data were analyzed using SPSS v16 software.Results: One hundred patients (44 men and 46 women) with a mean age of 52 ± 2 years participated in this study. Among them, 76 had pemphigus vulgaris, 18 had bullous pemphigoid, and 6 had pemphigus foliaceous. A median score of six was recorded for the ABQOL, and a median score of one was recorded for the ABSISscale. The relationship between quality of life and disease severity was statistically significant (P = 0.001). Also, a weak but statistically significant association was observed between the quality of life and patients’ age (P = 0.049).Conclusion: We demonstrated that increased disease severity significantly impairs AIBD patients’ quality of life. On this account, patients with severe AIBDs require more social, psychological, and financial support.
Shrutakirthi D. Shenoi; Punya Suvarna; Shibani Bhatia; Sukriti Arora
Abstract
Pemphigus vulgaris is one of the most common autoimmune blistering disorders, with most patients presenting in late stages with severe and extensive erosions over the skin. Along with systemic management of the disease, it is important to provide local wound care to prevent secondary infection. Here, ...
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Pemphigus vulgaris is one of the most common autoimmune blistering disorders, with most patients presenting in late stages with severe and extensive erosions over the skin. Along with systemic management of the disease, it is important to provide local wound care to prevent secondary infection. Here, we report the case of a patient who presented with extensive erosions and showed no response to standard dressing protocols. Thus, it is important to review alternate, easily available, environmentfriendly dressing materials. This case report discusses how banana leaves and gentian violet were used to manage severe and extensive erosions in a pemphigus patient who showed no improvement with modern-day dressings. We also compared the costs of modern-day dressings with the banana leaf dressing used in our setup. Following the success of this methodology, we have been using banana leaves for various chronic non-healing wounds and ulcers.
zeinab Aryanian; Mohammad Keramatipour; Nafiseh Esmaili; Azadeh Goodarzi; Arghavan Azizpour; Ifa Etesami; Maede Rayati Damavandi; Ghazaleh Zarrinrad; Somayeh Ahmadloo; Narges Ghandi
Abstract
Background: Pemphigus vulgaris (PV) is an autoimmune bullous disease of the skin and mucous membranes caused by activation and proliferation of T cells, production of Th2 cytokine profile and pathogenic antibodies. Vitamin D is a probable immunodeviator to Th2, which its actions are ...
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Background: Pemphigus vulgaris (PV) is an autoimmune bullous disease of the skin and mucous membranes caused by activation and proliferation of T cells, production of Th2 cytokine profile and pathogenic antibodies. Vitamin D is a probable immunodeviator to Th2, which its actions are mediated through the vitamin D receptor (VDR). FokI is the only single nucleotide polymorphism (SNP) leading to VDR protein with a different structure and function. For the first time, we focused on FokI VDR SNP to evaluate its potential role in the genetic susceptibility to PV, particularly in the Iranian population that has a high prevalence of pemphigus.Methods: In this case-control study, DNA samples of 122 PV patients and 233 healthy controls were extracted, and FokI genotyping was performed using the PCR-RFLP method.Results: The mean allele frequencies of F and f alleles in the PV and control groups were 75% and 25%, and 78% and 22%, respectively, showing no significant difference. The genotype frequencies for FF, Ff, and ff genotypes in the case group were 57.4%, 35.2%, and 7.4%, respectively. In the control group, the frequencies were 60%, 36%, and 4%, respectively. Statistical analysis showed no significant difference between the two groups.Conclusion: The present study concluded the frequencies of F and f alleles as approximately 77% and 23% in the gene pool of the Iranian population. Additionally, it showed no association between the FokI alleles and PV in this population.
Bakhtiari Sedigheh; Toossi Parviz; Taheri Jamileh Beigom; Azimi Somayyeh; Bengar Mahnaz Kalantari; Namazi Zahra; Rafieian Nasrin
Volume 20, Issue 3 , 2017, , Pages 75-78
Abstract
Background: ABO blood group antigens may play a role in the pathophysiology of certain diseases. Several studies have investigated the relation between blood group and autoimmune disorders. In this research, we evaluated the associations between ABO blood types and rhesus (Rh) factor in pemphigus patients.Methods: ...
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Background: ABO blood group antigens may play a role in the pathophysiology of certain diseases. Several studies have investigated the relation between blood group and autoimmune disorders. In this research, we evaluated the associations between ABO blood types and rhesus (Rh) factor in pemphigus patients.Methods: In this case-control, hospital-based study, we evaluated the prevalence of blood group on 110 pemphigus patients and 126 controls. The chi-square test was used to compare qualitative variables and examine the relationship between blood groups and Rh in pemphigus patients and a control group.Results: Blood group (O) was found in 46.3% (O+ 38.2%) of patients with pemphigus, followed by blood type A (30.0%; A+ 27.3%), and blood groups B and AB. Compared to the control group, we noted that the distribution of blood groups, with Rh (P=0.73) or without Rh (P=0.52), did not significantly differ.Conclusion: Although some associations between blood group antigens and specific diseases have been reported in the literature, this study did not find any statistically significant relationship between blood groups and pemphigus disease.
Anchal Jindal; Rushikesh Shah; Neela Patel
Volume 17, Issue 3 , 2014, , Pages 96-100
Abstract
Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. The aim of our study was to analyze and correlate clinical, histopathological, and immunofluorescence findings in ...
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Background: Immunobullous diseases are morphologically heterogeneous and the differentiation between various subtypes is essential for proper treatment and prognosis. The aim of our study was to analyze and correlate clinical, histopathological, and immunofluorescence findings in autoimmune bullous diseases.Method: A cross-sectional study was conducted over a period of two years (2010-2012) after receiving the ethics committee approval. Sixty patients who met the inclusion criteria of immunobullous disease were included in the study. Skin biopsy for histopathological and direct immunofluorescence (DIF) examination was taken. DIF was also done in a few of the cases using the salt-split technique. The final diagnosis was based on clinical, histopathological, and DIFfindings.Result: Sixty cases with a clinical diagnosis of autoimmune bullous diseases were evaluated. In 95% of the cases, the histopathological diagnosis was consistent with the clinical diagnosis and in 75% of the cases, the DIF diagnosis was consistent with the clinical diagnosis. A positive relationship was seen between clinical and DIF findings with r = 0.67 and between histopathologic and DIF findings with r = 0.76. DIF positivity was seen in 100% of the cases of bullous pemphigoid (BP), 100% of the cases of pemphigus foliaceus, and 94.7% of the cases of pemphigus vulgaris which was statistically significant with P < 0.05.Conclusion: Our study provides evidence-based guidance for the diagnosis and classification of various immunobullous disorders. DIF test should be done in conjunction with histopathology tomake a definite diagnosis and minimize both false-positive and false-negative results.
Handjani Farhad; Kalafi Amir
Volume 16, Issue 4 , 2013, , Pages 128-131
Abstract
Background: Chronic skin diseases such as vitiligo and psoriasis can impair the quality of life in the patients with these diseases. The impact of chronic and/or severe skin diseases on the lives of family members of the affected patients has only recently been addressed. The tool used for this assessment ...
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Background: Chronic skin diseases such as vitiligo and psoriasis can impair the quality of life in the patients with these diseases. The impact of chronic and/or severe skin diseases on the lives of family members of the affected patients has only recently been addressed. The tool used for this assessment is the Family Dermatology Life Quality Index (FDLQI). The aim of this study was to use the FDLQI to assess the impact of four skin diseases (pemphigus, psoriasis, vitiligo, and cutaneous leishmaniasis) on family members of the affected patients.Method: Fifty healthy family members of 50 patients with pemphigus, psoriasis, vitiligo, and cutaneous leishmaniasis were included in this study. The patients had to have an extensive disease with disease duration of at least one month. One family member of each patient completed the 10- item Persian version of the FLDQI questionnaire. The score of this index ranged from 0 to 30.Result: The mean duration and mean FDLQI score of the diseases were as follows: psoriasis (mean duration=4.1±2.7, mean FDLQI score=14.7±5.01), pemphigus (mean duration=3.6±2.3, mean FDLQI score=15.4±4.7), vitiligo (mean duration=5.1±4.05, mean FDLQI score=14.4±5.08) and cutaneous leishmaniasis (mean duration=0.62±0.23, mean FDLQI score=12.0±4.3). There was no significant difference among the groups (p=0.562). In addition, no strong correlation was observed between the mean duration of the disease and mean FDLQI scores for each condition (r=0.051, p=0.726).Conclusion: This small-scale study demonstrated that although not statistically significant, inflammatory diseases like pemphigus had a higher FDLQI score when compared to non-inflammatory conditions like vitiligo and short-term conditions such as leishmaniasis.
Layegh Pouran; Nahidi Yalda; Malekzadeh Iman; Shakeri Mohammad Taghi
Volume 16, Issue 3 , 2013, , Pages 100-104
Abstract
Background: Pemphigus is a rare autoimmune disease causedby autoantibodies against desmoglein. It clinically presents withpainful blisters and erosions on the skin and mucous membranes.Few studies have been conducted on the quality of life inpemphigus patients which have all indicated the strong impactof ...
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Background: Pemphigus is a rare autoimmune disease causedby autoantibodies against desmoglein. It clinically presents withpainful blisters and erosions on the skin and mucous membranes.Few studies have been conducted on the quality of life inpemphigus patients which have all indicated the strong impactof the disease on emotional and physical status of the patients.According to evident differences in the culture and quality oflife between our society and western societies, we assessed thequality of life in patients with pemphigus in the Northeast of Iran.Method: This study was conducted on 78 pemphigus vulgarispatients who were visited or hospitalized in the dermatologyclinics of Qaem and Imam Reza Hospitals in Mashhad, Iran.Data collection was done by the Dermatology Life Quality Index(DLQI) questionnaire in patients with pemphigus vulgaris.Statistical analysis was performed using SPSS 11.5 software withChi-square, T-test and ANOVA statistical tests.Result: The disease had a significant impact on the quality oflife in the majority of patients (31 (39.7%) patients). Hospitalizedand newly diagnosed cases and those receiving higher doses ofsteroid had lower quality of life. The patient’s age, sex, educationlevel, and therapeutic regimen did not have a significant effecton the quality of life.Conclusion: Pemphigus is not just a physical disease and hasvarious aspects. It is responsible for many changes in healthrelatedquality of life in patients. Therefore, we will be successfulin treating this disease only when psychological and social aspectsof pemphigus are considered in addition to clinical improvementof the patients.
Mahnaz Banihashemi; Masoud Maleki; Fakhrozaman Pezeshkpoor; Amir Hossein Jafarian; Mohammad Reza Sharghi; Sara Hashemzadeh
Volume 16, Issue 2 , 2013, , Pages 53-56
Abstract
Background: Pemphigus is a group of autoimmune blistering skin diseases that is related to auto antibodies against desmoglein 1 and 3. Many reports have shown that HSV1, HSV2, VZV, EBV, CMV, HHV8, and HIV are triggering agents for the activation and exacerbation of pemphigus. In this ...
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Background: Pemphigus is a group of autoimmune blistering skin diseases that is related to auto antibodies against desmoglein 1 and 3. Many reports have shown that HSV1, HSV2, VZV, EBV, CMV, HHV8, and HIV are triggering agents for the activation and exacerbation of pemphigus. In this study, we decided to evaluate the frequency of HSV1, HSV2, HHV8, and EBV in paraffin-embedded specimens of the new cases of pemphigus patients using immunohistochemical methods.Method: Thirty patients with pemphigus whose diagnosis was proved with direct immunofluorescens studies (20 cases of pemphigus vulgaris and 10 cases of pemphigus foliaceus) and 10 specimens from the free margins of excised melanocytic nevi were collected. Immunohistochemical staining was performed for HSV1 and EBV (using Novo Castra kit) and for HSV2 and HHV8 (using Dako kit).Result: The results showed a significance difference in the frequency of positive staining for HSV1 in skin lesions of pemphigus vulgaris, foliaceus, and controls (P= 0.041), foliaceus group more positive staining than vulgaris group and both more than controls, but not for HSV2, EBV, and HHV8 markers. Positive staining for HHV8 occurred in 30% of PF patients and 15% of PV patientsbut P value was not significant (0.171).Conclusion: Like previous studies, our research also indicated significant prevalence of HSV1 in lesions of pemphigus patients, especially in pemphigus foliaceus. We collected new cases ofpemphigus before starting immunosuppressive therapy, so we think that the presence of HSV1 in skin lesions of patients can act as a triggering factor for the disease which could not be attributed to the suppressive therapy.
Barzegari Massoume; Valikhani Mahin; Esmaili Nafiseh; Naraghi Zahra; Nikoo Azita; Kamyab Kambiz; Ghanadan Ali; Tamizifar Banafshe
Volume 11, Issue 2 , 2008, , Pages 64-66
Abstract
Background: Pemphigus vulgaris (PV) is an autoimmune blistering disease, caused by autoantibodies against desmoglein (Dsg) 3 and / or Dsg1 which induce the loss of adhesion between keratinocytes. Nikolsky's sign is the ability to induce peripheral extension of a blister as a consequence of applying lateral ...
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Background: Pemphigus vulgaris (PV) is an autoimmune blistering disease, caused by autoantibodies against desmoglein (Dsg) 3 and / or Dsg1 which induce the loss of adhesion between keratinocytes. Nikolsky's sign is the ability to induce peripheral extension of a blister as a consequence of applying lateral pressure to the border of an intact blister. If the weakening of the intercellular adhesion is present but not marked, then the damage may be demonstrated only microscopically (microscopic Nikolsky’s sign and can increase the sensitivity of the histopathological studies. Methods: We studied 40 patients and divided them randomly into two groups (A, B). Group A were subjected to the tangential pressure over the perilesional skin before a biopsy specimen was taken from that site; group B patients were subjected to a biopsy without the tangential pressure technique. Results: Histopathological changes of pemphigus vulgaris were present in 30% of the patients in group A and 5% of the patients in group B. They were not statistically different. The presence of microscopic Nikolsky’s sign was significantly higher in patients with generalized disease. Conclusion: Microscopic Nikolsky sign can increase the sensitivity of histologic diagnosis of PV.
Samadi Zaynab; Gorouhi Farzam; Davari Parastoo; Firooz Alireza
Volume 9, Issue 4 , 2006, , Pages 335-342
Abstract
Background and aim: Pemphigus vulgaris (PV) is the most common blistering disease in Iran and many other Asian countries with a relatively high incidence and involvement of both skin and mucous membrances in majority of patients. The aim of this study was to assess the opinions of Asian experts on the ...
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Background and aim: Pemphigus vulgaris (PV) is the most common blistering disease in Iran and many other Asian countries with a relatively high incidence and involvement of both skin and mucous membrances in majority of patients. The aim of this study was to assess the opinions of Asian experts on the diagnosis and management of PV. Materials and Methods: A questinnaire-based mailed/emailed survey was conducted. The questionnaire was sent to 29 dermatologists from different countries of Asia (23 from Iran and 6 from India, Kuwait, Turkey and Bangladesh) who are in charge of the treatment of autoimmune blistering disorders with at least 5 years experience in this field who visit at least five new PV patients annually. Questions incluced duration of experience, number of patients treated and diagnostic and treatment approaches for PV. Descriptive data were reported using SPSS 11.5 and some data are reported as mean±SD. Results: All 29 physicians participated in the survey. Among them, 79.3% visit their patients within 6 months after the onset of symptoms. Diagnosis of PV is confirmed by histologic and direct immunofluorescence examinations by 65.5% of physicians. All of them initiate the treatment with corticosteroids (48.3% with a dose of at least 2 mg/kg/day prednisolone) and 89.7% add adjuvant immunosuppressors at the same time. Of the adjuvant agents used, azathioprine is used by 82.8% of physicians. Conclusion: Different trends in diagnostic techniques and treatment options for PV among the experienced authorities emphasize the urgent need for large-scale controlled trials to reach consensus standards in this field. In addition, regional and worldwide consensus meetings to consider all regional and genetic similarities and differences are highly recommended.
Z Hallaji; Sh Shams Davatchi; M Vali Khani; N Ghandi; M Kar Bakhsh Davari; M Hedayati; AR Firooz
Volume 9, Issue 3 , 2006, , Pages 204-210
Abstract
Background and aim: Azathioprine is the most widely used immunosuppressive agent as an adjunct to corticosteroids in the treatment of pemphigus vulgaris (PV). Thiopurine methyl transferase (TPMT) is a key enzyme in azathioprine metabolism and a genetic polymorphism controls its activity in human tissue. ...
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Background and aim: Azathioprine is the most widely used immunosuppressive agent as an adjunct to corticosteroids in the treatment of pemphigus vulgaris (PV). Thiopurine methyl transferase (TPMT) is a key enzyme in azathioprine metabolism and a genetic polymorphism controls its activity in human tissue. TPMT activity can provide a rational basis to determine suitable dose of azathioprine, theoretically. The aim of this study was to evaluate the clinical relevancy of this hypothesis in PV patients.Materials and Methods: In this cross sectional study in Razi Hospital, the activity of TPMT in the red blood cells of 52 PV patients who received azathioprine for at least 12 months and 29 PV patients who did not receive this drug was measured and correlated to the clinical response and side effects observed.Results:The mean of TPMT activity was not significantly different in patients with unfavourable response, comparing to patients with favorable response to azathioprine (P=0.087). No relationship was observed between total dose of corticosteroid and TPMT activity (r=0.089, P=0.583). There was no difference between the mean of TPMT activity in patients receiving azathioprine and those not receiving this drug (P=0.36).Conclusion: A direct relationship was not observed between TPMT activity and clinical efficacy and side effects in PV patients under treatment with azathioprine. Larger prospective studies in more homogenous patients are needed to evaluate the clinical relevance of TPMT polymorphism and to determine accurate azathioprine dosing guidelines based on TPMT activity.
A Asilian; AR Yousefi
Volume 8, suppl , 2004, , Pages 47-52
Abstract
Pemphigus is a chronic blistering mucocutaneous disease, classified in immunobullous skin diseases. Infections, particularly septicemia due to Staphylococcus aureus and Pseudomonas aeroginosa are the most common and dangerous disorders due to immunosuppressive therapy in this disease. Infection with ...
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Pemphigus is a chronic blistering mucocutaneous disease, classified in immunobullous skin diseases. Infections, particularly septicemia due to Staphylococcus aureus and Pseudomonas aeroginosa are the most common and dangerous disorders due to immunosuppressive therapy in this disease. Infection with nocardia is rare, so that only 6 cases have been reported yet. Herein, we report a 56-year-old man with one-year history of pemphigus treated with prednisolone and cyclophosphamide, who at the same time developed skin and pulmonary nocardiosis. He had a good response to treatment with cotrimoxazole.
R Yaghoobi; M Omidian; N Sina
Volume 4, Issue 3 , 2001, , Pages 50-53
Abstract
Pemphigus is rare in children, only 71 cases have been reported in literature. We report 2 cases of pemphigus vulgaris (13 and 15 years old) among 147 cases of pemphigus from Khouzestan who were admitted to the Department of Dermatology, Ahwaz Medical University between 1989-2001. The basis of diagnosis ...
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Pemphigus is rare in children, only 71 cases have been reported in literature. We report 2 cases of pemphigus vulgaris (13 and 15 years old) among 147 cases of pemphigus from Khouzestan who were admitted to the Department of Dermatology, Ahwaz Medical University between 1989-2001. The basis of diagnosis was clinical and histopathological findings in each patient. One patient was treated only with oral corticosteroid, and in the other patient dapson was added for steroid-sparing effect. Long-term follow-up is necessary to detect fairing of the disease and side effects of immunosuppressive drugs.
R Yaghoobi
Volume 3, Issue 1 , 1999, , Pages 16-23
Abstract
Background: Pemphigus is an autoimmune disease characterized with intra-epidermal cleavage. Objective: The aim of this study was to examine the clinical and epidemiologic features of pemphigus in Khouzestan province, southwest Iran. Patients and Methods: All new cases of pemphigus admitted in the department ...
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Background: Pemphigus is an autoimmune disease characterized with intra-epidermal cleavage. Objective: The aim of this study was to examine the clinical and epidemiologic features of pemphigus in Khouzestan province, southwest Iran. Patients and Methods: All new cases of pemphigus admitted in the department of dermatology, Sina Hospital, Ahwaz University from October 1990 to September 1999 were studied. The diagnosis of pemphigus was based on clinical and histological findings. Results: One hundred-eleven pemphigus patients represented 4.7% of 2358 patients admitted to this department in this period. The most common presentation was pemphigus vulgaris (81.98%), followed by pemphigus foliaceous (18.02%). Pemphigus was observed more common in women (62 of 111; 55.86%) than in men (49 of 111; 44.14%). The mean age of all variants of pemphigus and in both sex at the time of diagnosis was 42.5 years. The lag time between the onset of illness and final diagnosis in pemphigus vulgaris and pemphigus foliaceous were 4 and 9 months, respectively. In pemphigus vulgaris patients, the onset of lesions were 54.65% and 45.34% from mucous membranes (Mainly oral cavity) and skin in 54.65% and 45.35% of cases, respectively. In all of pemphigus foliaceous patients, the onset of lesions was from the skin. The mortality was 6.30%, more in pemphigus foliaceous than vulgaris. Conclusion: This study showed that pemphigus is relatively common in Khouzestan province, compared with other regions. Pemphigus vulgaris is the most common variant and mortality was higher in pemphigus foliaceous.